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rapidly progressive dementia continuum

Paraneoplastic limbic encephalitis (Acute limbic encephalitis. Brain autopsy revealed hypoxic/hypoglycemic nerve loss in multiple brain regions (cortices, CA1/CA4 hippocampus, deep nuclei, cerebellar Purkinje cells, and dentate nucleus) without evidence of prion disease.Modified with permission from Rosenbloom MH, et al, Neurol Clin Pract.A 50-year-old right-handed man with a history of alcoholism (current), hypertension, hypercholesterolemia, and hepatitis C developed nausea, vomiting, and diarrhea for 3 days. eds. The first symptoms of a dementia can help with diagnosis. Metabolic disorders with clinical and radiologic features of sporadic Creutzfeldt-Jakob disease. Chitravas N, Jung RS, Kofskey DM, et al. Zeidman SM, Monsein LH, Arosarena O, et al. Rapidly progressive dementia. Prion diseases are rare, with an incidence of 1.2 in 1 million, but should be considered in the differential diagnosis. A 59-year-old woman developed confusion, progressive aphasia, mutism, and fluctuations of alertness over 2 weeks. 2018;153:371-397. doi: 10.1016/B978-0-444-63945-5.00020-9.Psychiatr Clin North Am. The differential diagnosis and clinical workup for rapidly progressive dementia are quite extensive and involve searching for infectious, inflammatory, autoimmune, neoplastic, metabolic, and neurodegenerative causes. They constitute the second largest group of rapidly progressive dementias in 2 large cohorts but are a much smaller portion of another group of presenile rapid dementias.Nearly 1 in 20 patients with MS manifest dementia, but the prevalence of other cognitive disturbances is much more common and subtle.This condition related to Hashimoto thyroiditis is also referred to as steroid-responsive encephalopathy with autoimmune thyroiditis, which explains the nature of this syndrome that is believed to be related to a cross-reaction between autoimmune antithyroid antibodies and neurons, resulting in generalized CNS inflammation and neurologic impairment.As a systemic granulomatous disease, sarcoidosis may simulate many neurologic disorders, and likewise, imaging of neurosarcoidosis is complex.Primary CNS vasculitis is a rare entity, but systemic vasculitides may also present with CNS manifestations with cognitive deficits in half of patients.Celiac disease, an intolerance of food products containing gluten, often with the presence of autoantibodies to the enzyme tissue transglutaminase, may present with neurologic symptoms, including dementia, though those reported are quite varied, ranging from chronic headaches to ataxia.There are a number of paraneoplastic neurologic disorders with a diverse set of clinical presentations that may present with cognitive impairment or dementia. 2020 Jun;139(6):1045-1070. doi: 10.1007/s00401-020-02148-4. Both the clinician and radiologist alike should be open to the possibility of alternate diagnoses in the setting of rapidly progressive presenile dementias, particularly because several diseases within this group benefit from urgent and specific treatment.Thank you for your interest in spreading the word on American Journal of Neuroradiology.NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. Although American Academy of Neurology (AAN) guidelines currently do not recommend rapid plasma reagin (RPR) as a screening test for dementia, at the UCSF center the author prefers to send this test, as it is a relatively inexpensive test for a treatable dementia. The role of imaging in this grouping of conditions is much more problematic than in the aforementioned diagnoses because imaging findings, if present, are generally nonspecific and may only offer a glimpse of the correct diagnosis, which may be more readily obtained with thorough history-taking and laboratory studies.Most cases of neurotoxic exposure are clinically marked by fulminant encephalopathic symptoms of acute onset with a clear source rather than by symptoms of a rapidly progressive dementia. Pitsavas S, Andreou C, Bascialla F, et al. Neurology 2011; 76(20): 1711–1719. April 2016, Vol.22, No.2 Hurst RW, Bagley LJ, Galetta S, et al. Brain imaging usually is done prior to CSF analysis as it might direct what CSF tests are sent, as well as rule out a space-occupying lesion. Major diagnostic categories of patients with rapidly progressive dementia (RPD) referred to, versus… Algorithm for evaluating rapidly progressive dementia.

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